Hyperlipidemia
Classification and external resources
ICD-10	E78.
ICD-9	272.0-272.4
DiseasesDB	6255
MeSH	D006949

Hyperlipidemia, hyperlipoproteinemia, dyslipidemia or hyperlipidaemia (British English) is the presence of raised or abnormal levels of lipids and/or lipoproteins in the blood. Lipids (fatty molecules) are transported in a protein capsule, and the density of the lipids and type of protein determines the fate of the particle and its influence on metabolism.

Lipid and lipoprotein abnormalities are extremely common in the general population, and are regarded as a highly modifiable risk factor for cardiovascular disease due to the influence of cholesterol, one of the most clinically relevant lipid substances, on atherosclerosis. In addition, some forms may predispose to acute pancreatitis.

Contents 1 Classification 1.1 Hyperlipoproteinemia type I 1.2 Hyperlipoproteinemia type II 1.2.1 Type IIa 1.2.2 Type IIb 1.3 Hyperlipoproteinemia type III 1.4 Hyperlipoproteinemia type IV 1.5 Hyperlipoproteinemia type V 2 Unclassified forms 3 Treatment 4 See also 5 References 6 External links

[edit] Classification

Hyperlipidemias are classified according to the Fredrickson classification which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation.^[1] It was later adopted by the World Health Organization (WHO). It does not directly account for HDL, and it does not distinguish among the different genes that may be partially responsible for some of these conditions. It remains a popular system of classification, but is considered dated by many.

Fredrickson classification of Hyperlipidemias

Hyperlipoproteinemia	OMIM	Synonyms	Defect	Increased lipoprotein	Treatment	Serum appearance
Type I (rare)	238600	Buerger-Gruetz syndrome, Primary hyperlipoproteinaemia, or Familial hyperchylomicronemia	Decreased lipoprotein lipase (LPL) or altered ApoC2	Chylomicrons	Diet control	Creamy top layer
Type IIa	144400	Polygenic hypercholesterolaemia or Familial hypercholesterolemia	LDL receptor deficiency	LDL	Bile acid sequestrants, statins, niacin	Clear
Type IIb	144400	Combined hyperlipidemia	Decreased LDL receptor and increased ApoB	LDL and VLDL	Statins, niacin, fibrate	Clear
Type III (rare)	107741	Familial dysbetalipoproteinemia	Defect in Apo E 2 synthesis	IDL	Fibrates, statins	Turbid
Type IV	144600	Familial hyperlipemia	Increased VLDL production and Decreased elimination	VLDL	Fibrate, niacin], statins	Turbid
Type V (rare)	144650	Endogenous hypertriglyceridemia	Increased VLDL production and Decreased LPL	VLDL and Chylomicrons	Niacin, fibrate	Creamy top layer & turbid bottom

[edit] Hyperlipoproteinemia type I

Type I hyperlipoproteinemia is a form of hyperlipoproteinemia associated with deficiencies of lipoprotein lipase.

[edit] Hyperlipoproteinemia type II

Hyperlipoproteinemia type II, by far the most common form, is further classified into type IIa and type IIb, depending mainly on whether there is elevation in the triglyceride level in addition to LDL cholesterol.

[edit] Type IIa

This may be sporadic (due to dietary factors), polygenic, or truly familial as a result of a mutation either in the LDL receptor gene on chromosome 19 (0.2% of the population) or the ApoB gene (0.2%). The familial form is characterized by tendon xanthoma, xanthelasma and premature cardiovascular disease. The incidence of this disease is about 1 in 500 for heterozygotes, and 1 in 1,000,000 for homozygotes.

[edit] Type IIb

The high VLDL levels are due to overproduction of substrates, including triglycerides, acetyl CoA, and an increase in B-100 synthesis. They may also be caused by the decreased clearance of LDL. Prevalence in the population is 10%.

• Familial combined hyperlipoproteinemia (FCH)
• Secondary combined hyperlipoproteinemia (usually in the context of metabolic syndrome, for which it is a diagnostic criterion)

[edit] Hyperlipoproteinemia type III

This form is due to high chylomicrons and IDL (intermediate density lipoprotein). Also known as broad beta disease or dysbetalipoproteinemia, the most common cause for this form is the presence of ApoE E2/E2 genotype. It is due to cholesterol-rich VLDL (β-VLDL). Prevalence is 0.02% of the population.

[edit] Hyperlipoproteinemia type IV

This form is due to high triglycerides. It is also known as hypertriglyceridemia (or pure hypertriglyceridemia). According to the NCEP-ATPIII definition of high triglycerides (>200 mg/dl), prevalence is about 16% of adult population.^[2]

[edit] Hyperlipoproteinemia type V

This type is very similar to type I, but with high VLDL in addition to chylomicrons.

It is also associated with glucose intolerance and hyperuricemia

[edit] Unclassified forms

Non-classified forms are extremely rare:

• Hypo-alpha lipoproteinemia
• Hypo-beta lipoproteinemia (prevalence 0.01-0.1%)

[edit] Treatment

Please help improve this article by expanding it. Further information might be found on the talk page. (March 2009)

For treatment of type II, dietary modification is the initial approach but many patients require treatment with statins (HMG-CoA reductase inhibitors) to reduce cardiovascular risk. If the triglyceride level is markedly raised, fibrates may be preferable due to their beneficial effects. Combination treatment of statins and fibrates, while highly effective, causes a markedly increased risk of myopathy and rhabdomyolysis and is therefore only done under close supervision. Other agents commonly added to statins are ezetimibe, niacin and bile acid sequestrants. There is some evidence for benefit of plant sterol-containing products and ω₃-fatty acids^[3]

[edit] See also

• Primary hyperlipoproteinemia

[edit] References

[edit] External links

• The Fredrickson papers (with photos from early lipoprotein research)
• 745209914 at GPnotebook

v • d • e Inborn error of lipid metabolism: dyslipidemia (E78, 272.0-272.6) Hyperlipidemia Hypercholesterolemia/Hypertriglyceridemia (Type I, Familial hypercholesterolemia/Type IIa, Combined hyperlipidemia/Type IIb) · Xanthoma Hypolipoproteinemia Hypoalphalipoproteinemia/HDL Lecithin cholesterol acyltransferase deficiency · Tangier disease Hypobetalipoproteinemia/LDL Abetalipoproteinemia · Apolipoprotein B deficiency Lipodystrophy Barraquer-Simons syndrome Other Lipomatosis · Adiposis dolorosa · Lipoid proteinosis see also lipoprotein metabolism